New Defibrillators Keep 5 Family Members From Cardiac Arrest

May 5, 2011 — -- Members of LaNay Miceli's family seemed to be dying, young and suddenly, all around her. There was her 24-year-old cousin who just didn't wake up one morning, her 55-year-old cousin who went into cardiac arrest while chatting on the telephone and another cousin who was only 40 when he had a heart attack. And that was just last year.

In the past, three of Miceli's other cousins also died unexpectedly -- one at 35, another at 39 and the last at 52. Her mother's brother died of a heart attack at 36.

"I thought something was odd," said Miceli, 53, a sanitary worker for the city of Chicago. "Too many people kept passing away."

An explanation came only when Miceli's 22-year-old cousin underwent medical tests that revealed Long QT syndrome, a rare genetic heart rhythm disorder that causes fast and chaotic heartbeats. They can trigger fainting spells, seizures, and even sudden death.

Dr. Bradley Knight, director of cardiac electrophysiology at Northwestern Memorial Hospital in Chicago, reached Miceli, suggesting that she and her family be tested as well.

The results were sobering.

"I have it, but my sister doesn't," said Miceli. "My two children had to be tested and they both have it, and my daughter's two boys were both diagnosed, as well."

No one in the family had ever had symptoms or indicators of heart arrhythmia.

"It was a little overwhelming to hear [the diagnosis], but we kept it in perspective," said Miceli. "We don't have a death sentence because we had the device put in."

Miceli, her children and cousin decided to have heart defibrillators implanted in their bodies to prevent cardiac arrest.

"In general, the patients who are at high risk of sudden cardiac arrest are older patients with heart disease," Knight said. "However, a small number of these patients have primary electrical problems of the heart. These can even be pediatric patients."

Doctors said Miceli's grandchildren, aged 5 and 1, are too young to have the devices implanted, and instead were given beta blockers, medications they take three times a day.

The devices, called implantable cardioverter defibrillators -- ICDs for short -- save lives by detecting and correcting abnormal heart rhythms that can lead to cardiac arrest and death. An ICD includes a battery pack that is typically placed under the collar bone, with wires that run through the veins and into the heart, usually through the right ventricle. The device acts as an emergency response that signals and restores an irregular heartbeat if necessary.

"In young patients, the [older model] can be a real problem because the wires we have are imperfect and because the wires in the blood vessel are subjected to stress and trauma from the heart beating," said Dr. Kenneth Ellenbogen, chairman of the division of cardiology at Virginia Commonwealth University School of Medicine. "Every time the heart beats, it's being stretched and pulled, and over a period of time, there is a risk that the wires will fracture or break."

To avoid this problem, Miceli and her family opted for a new model on which Knight is conducting human trials. It is under review with the FDA.

Researchers formally presented the new device on Wednesday at the Heart Rhythm Society's 32nd Annual Scientific Sessions. The defibrillator, known as S-ICD, is implanted just under the skin, on the chest wall, below the breast tissue. Similar to the old model, scientists said that it provides protection against abnormal heart rhythms, but it is less invasive because it does not require wires to touch the heart.

The device is already in use in Europe and it is currently available through clinical trials in the United States.